2 of these disease are seen often on both the heme consult service and boards.
Cold Agglutinin Disease
Hemolytic anemia precipitated by cold
Monoclonal IgM binds RBCs (I antigen), agglutination, and fixes complement in cold->hemolysis
DAT+ for C3 and - for IgG
Associated with IgM gammopathies/LPL
Polyclonal form after Mycoplasma infection
Rx = Rituximab
Pathological iron deposition in mitochondria of erythroid precursors
Pathologic finding
🚫a specific diagnosis!
Broad classification of sideroblastic anemias.
1. Congenital
-Syndromic
-Non-syndromic
2. Acquired
-Clonal
-Metabolic
-Drugs
WHO defined types of sideroblasts
Type 1: <5 siderotic granules in cytoplasm.
Type 2: ≥5 granules but no perinuclear distribution.
Type 3 = ringed sideroblast: ≥ 5 granules in a perinuclear position, surrounding nucleus or encompassing one third of nuclear circumference.